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تصنيف المرضى العراقيين المصابين بالاورام العصبيه الغديه وتقدير مدى استجابتهم لعقارالساندوستاتين (الاوكتيريوتايد) == NEUROENDOCRINE TUMORS CLASSIFICATION AND RESPONSE TO LONG ACTING SOMATOSTATIN ANALOGUE; AN IRAQI EXPERIENCE
Author name:
مروة خالد عبد الفتاح
Supervisor name:
منور عبد الاله النقاش
General topic:
Medicine
Specific topic:
Therapeutic Radiology
Degree:
Higher Diploma
University:
University of Baghdad - Faculty Of Medicine
Language:
English
University location:
Baghdad
First pages:
19T1531 - p.pdf
Abstract:
The lack of studies regarding the incidence of neuroendocrine tumors (NETs) is related to the rarity of these tumors (25/1,000,000). Chromogranin A is a useful tumor marker for NETs diagnosis & follow - up. Octereotide LAR is an established treatment for NETs by both providing symptomatic relief & inhibiting tumor growth. However, studies regarding incidence of NETs & their response to SAS - LAR are still insufficient.Aim of studyTo shade the light on the incidence of NETs, the clinico - pathologic characteristics of Iraqi patients with NETs & their response to long acting Octereotide.Patients & methodsThis is a cross - sectional observational study that was conducted in Oncology teaching hospital/medical city complex using patients’ follow - up sheets. Data recruitment included all NET patients diagnosed after October 2013 to April 2016.ResultsIn our registry, 38 patients were recorded; most patients (60.5%) were over the age of 50 years with male to female ratio (1.2 : 1). We found the gastroenteropancreatic tract being the most common primary organ followed by pelvis . With respect to the GI tract we found that pancreas (26.3 %) was the commonest primary organ. Metastatic disease at presentation was found in (44.7%) of patients with the Liver (15.8%)was the most common metastatic site.Notably, most of our patients presented with G3 disease (44.7%) & (13.2%) with G1 . Serial CgA tests were performed in (17/28) patients used SAS - LAR, with a change in mean value from (225.3 U/L) pre - using the agent to (17.5 U/L) two months after use & to (8.7 U/L) four months after use(p=0.001) while the change in mean of CgA level was from (205.9 U/L) to (200.9 U/L) in 10 patients who did not use octereotide.(p=0.2). After a period of 2 years ,the median time of remission following SAS - LAR administration was 3 months compared to 7 months in patients received other modalities of treatment. In our study, a statistically significant difference was demonstrated between the two groups of patients.(p=0.003).Conclusion Plasma CgA is the most reliable marker for NETs, reflecting the clinical evolution of the disease. Aids in diagnosis & response assessment to different therapies.Octreotide LAR provides symptomatic response & contributes to disease stabilization & tumor regression in both functional & non functional NETs.1 - INTRODUCTION Neuroendocrine tumors are thought to arise from cells throughout the diffuse neuroendocrine system that is composed of peptide - and amine - producing cells that may secrete different hormones depending on the site of origin. NETs are composed of monotonous sheets of small round blue cells with uniform nuclei and cytoplasm(1). They compromise a broad family of tumors, the most common of which are carcinoid tumors (most commonly arise in the lungs & bronchi, small intestine, appendix rectum, or thymus)& pancreatic NETOther neuroendocrine tumors arise from parathyroid, adrenal, pituitary gland, & in calcitonin - producing cells of the thyroid (causing medullary thyroid carcinoma)( 2).Most NETs seem to be sporadic; risk factors for sporadic NET are poorly understood. Risk factors for the development of midgut carcinoid tumors include age, male sex, and increased body mass index, and menopausal hormone therapy (1).NET may also arise in the context of inherited genetic syndromes, include multiple endocrine neoplasia (MEN) types 1& 2 (2).The incidence of gastrointestinal (GI) NETs is 6.2 per 100,000 populations and has been steadily increasing. The increasing incidence of NETs reportedIn many studies is likely multifactorial and includes increased awareness and improved endoscopic methods of detection.As these tumors are indolent and patients survive a long time, the prevalence is quite high, making them the second most prevalent GI tract tumor, second only to colon cancer. Some are clinically silent and have been detected only at autopsy (incidence 8%). Further ,patients with GI NETs have a higher risk of other noncarcinoid primary tumors. The overall 5 - year survival rate of all patients with GI NETs is 28.5%.(1) Of all NETs ∼25% are located in the respiratory tract. Typical carcinoids (TCs) comprise ∼1% - 2% and atypical carcinoids (ACs) only 0.1% - 0.2% of pulmonary neoplasms. According to the surveillance, epidemiology and end results program (SEER) database from 2003, the combined incidence has been 1.57/100 000 inhabitants . The prevalence of thymic NET is ∼3% of the total number of NETs at all sites. In the last SEER database, a reported incidence of thymic NETs is 0.02/100 000 population per year . They constitute ∼5% of all thymic tumors. Both bronchial and thymic NETs may be part of multiple endocrine neoplasia type 1 syndrome (MEN - 1, 5% - 15%). The median age at diagnosis for bronchial NETs is 64 years and for thymic NETs 59 years.(3) The incidence of G1NET increased from 2.0 to 3.0; there was a large increase in G2NET from 0.01 in 1990 to 0.2 in 2010, and of the G3 - LCNET from 0.01 to 1.8, respectively. In G3 - SCNET incidence in men decreased from 21.3 to 10.1, whereas in women it increased from 4.5 to 7.7.(4).
